• Clinical and Experimental Pediatrics
• /
• v.45 no.6
• /
• pp.790-795
• /
• 2002

Sporadic reports of hypernatremic dehydration in breastfed newborn infants have appeared in medical literature for at least 3 decades. We report the first case of hypernatremic dehydration resulting from inadequate breast-feeding in Korea. A 14-day old baby, born to a mentally retarded mother, was transferred to our hospital with a body weight loss of 460 g since birth(17%) and a serum sodium(Na) level of 179 mEq/L, after initial hydration at another hospital. On admission, a cardiac murmur was heard and an enlarged liver was palpated. Cardiac ultrasonogram revealed ventricular septal defect and ostium secundum atrial septal defect. During hydration, a seizure-like attack developed. Serum Na decreased to 135 mEq/L on the 5th day of admission. Brain ultrasonography and brain magnetic resonance image revealed no remarkable abnormalities. Electroencephalography was normal. She suffered from prerenal azotemia, hyperglycemia and disseminated intravascular coagulation at admission but was treated successfully. Heart failure was also controlled with dobutamine, diuretics and digoxin.

• Clinical and Experimental Pediatrics
• /
• v.46 no.8
• /
• pp.826-830
• /
• 2003

ARCI syndrome consists of arthrogryposis, renal tubular acidosis, cholestatic jaundice and icthyosis. We experienced an ARCI syndrome case with corpus callosum hypoplasia and atrial septal defect. This case had oral feeding difficulty, multiple joint contracture, renal tubular acidosis and neurogenic muscular atrophy at neonatal period. At two months of age, icthyosis and cholestatic jaundice were diagnosed. The case was hospitalized due to pneumonia at four months of age. Corpus callosum hypoplasia and atrial septal defect were detected. The case was treated with a mechanical ventilator because pneumonia was aggravated and respiratory failure occurred. The patient expired at five months of age.

• Journal of Chest Surgery
• /
• v.23 no.5
• /
• pp.962-967
• /
• 1990

The anomalous pulmonary venous return of the entire left lung was an extremely rare congenital anomaly. The reported surgical experience with correction of this disorder was limited. The 3-year-old female patient underwent an operation upon the unilateral total anomalous pulmonary venous return from the left lung, in which the left superior pulmonary vein drained into innominate vein and the left inferior pulmonary vein into the coronary sinus, in Yeungnam University Hospital. The symptoms were nonspecific except frequent upper respiratory infection. Cyanosis was not seen. On auscultatory findings, a grade 2/6 systolic ejection murmur was audible over left second intercostal space of left sternal border and second heart sound had an increased pulmonary component which was widely splitted. The electrocardiogram demonstrated a right ventricular hypertrophy and right axis deviation and chest X-ray showed slightly increased pulmonary vascularity and bulged pulmonary conus. The echocardiogram demonstrated increased right atrial, ventricular, and pulmonary arterial dimension, and also secundum atrial septal defect and enlarged coronary sinus. The cardiac catheterization confirmed the left-to-right with a Qp/Qs of 2.0: 1 and oxygen step-up was seen in pulmonary artery, right ventricle, right atrium, and left innominate vein, and the catheter was not been introduced into the left pulmonary vein. A median sternotomy incision was done. Left superior pulmonary vein was drained to the innominate vein through anomalous vertical vein and the left inferior pulmonary vein drained to right atrium through the coronary sinus. The diversion of the left inferior pulmonary vein to posterior wall of left atrium was done after division in the proximity of coronary sinus. The anomalous vertical vein was diverted to base of left atrial auricle and then a atrial septal defect was sutured directly. The postoperative course was uneventful and she was discharged on the eleventh postoperative day. In the postoperative follow-up-2 months, she has been well without specific problems.

• Journal of Chest Surgery
• /
• v.24 no.1
• /
• pp.15-25
• /
• 1991

From February 1988 to December 1990, 42 patients underwent so called REV operation for pulmonary stenosis or atresia with or without anomalies of ventriculoarterial connection and truncus arteriosus. The principles of operative technique are mobilization of pulmonary arterial tree beyond the pericardial reflection, transection of pulmonary trunk between the pulmonary ventricle and pulmonary artery, suture of distal pulmonary arterial stump to the upper margin of Pulmonary ventriculotomy site with absorbable suture, and anterior patch with 0.625% glutaraldehyde fixed autologous pericardium with monocusp inside it. Age at operation ranged 3-156months [mean 41.8 month] with twelve of whom infants. Operative indications were pulmonary atresia, with ventricular septal defect[16], and pulmonary stenosis with double outlet right ventricle[8], with ventricular septal defect[16], with double outlet right ventricle[8], with complete transposition of the great arteries[8], with corrected transposition of the great arteries[6], with Fallot`s tetralogy[3], and truncus arteriosus[1]. There were six hospital deaths[14%] and no late death. Twenty-four of 36 survivals were followed up more than 12 months with good clinical results. Postoperative angiocardiogram was performed in fifteen patients. Hemodynamically, two patents had residual pressure gradients along the pulmonary outflow tract, one patient showed severe pulmonary regurgitation; morphologically, there were six significant stenosis of left pulmonary arterial tree, two of whom showed significant pressure gradients. Our present experience with REV operation suggests that this technique make it possible to perform anatomic repair in a wide variety of congenital anomalies of abnormal ventriculoarterial connection associated with pulmonary outflow tract obstruction without using the prosthetic material, even in infants, with relatively low mortality and morbidity.

• Journal of Yeungnam Medical Science
• /
• v.23 no.1
• /
• pp.19-25
• /
• 2006

Background: There is limited data on comparisons between the effect of histidin-etryptophan-ketoglutarate (HTK) solution and cold blood cardioplegic (CBC) solution in pediatric cardiac surgery. The purpose of this study was to compare the myocardial protective effect of HTK solution and CBC solution in patients undergoing pediatric cardiac surgery. Materials and Methods: We selected 49 patients with ventricular septal defect and atrial septal defect. HTK solution was used in 21 patients and CBC solution was used in 28 patents. HTK solution was given as a single dose, whereas CBC solution was used in the usual multi-dose method. The incidence of EKG change and concentration of Troponin T and CK-MB were compared for the evaluation of myocardial damage. Results: There were no significant differences in the incidence of ST, T segment change by EKG and serial cardiac enzyme levels between two groups. Conclusion: These results suggested that the myocardial protective effect of HTK solution was similar to CBC solution in simple pediatric cardiac surgery.

• Journal of Chest Surgery
• /
• v.32 no.2
• /
• pp.119-123
• /
• 1999

Background: From January 1989 to December 1996, we analyzed 22 cases of ventricular septal defect associated(VSD) with aortic valvular prolapse. Material and Method: The mean age of the patients was 7 years with a range of 6 months to 22 years . Thirteen patients were male and 9 were female. The types of VSD were Kirklin type I in 13 , Kirklin type II in 8 and Kirklin type I+II in one. Result: The preoperative echocardiographic findings were aortic valvular prolapse in 10 patients, aortic valvular prolapse associated with aortic regurgitation in 6, and only aortic regurgitation in 2. Aortic valvular prolapse were found in operation field in 4 that was not be in preoperative echcardiography. Preoperative mean Qp/Qs, systolic PAP, systolic RVP were 1.48${\pm}$0.42, 27.9${\pm}$9.87, 32.9${\pm}$10.87 mmHg, respectively. Twenty patients underwent patch closure of VSD, and two patients with moderate aortic regurgitation and prolapsed of the aortic valve underwent patch closure of VSD and aortic valvuloplasty. Short and long term echocardiographic follow-up in 8 patients who had preoperative aortic regurgitation were found to have improved or not aggravated by performing VSD patch closure only and patch closure with valvuloplasty in 2. Twelve patients who had only preoperative aortic valvular prolapse had no change in prolapsed valve in postoperative echocardiography. Conclusion: Early closure of VSD with patch is necessary in VSD with aortic valvular prolapse even in associated with mild regurgitation. But in moderate regurgitation, VSD closure with valvuloplasty is recommended.

• Journal of Chest Surgery
• /
• v.39 no.2 s.259
• /
• pp.154-156
• /
• 2006

Inferior sinus venosus ASD (atrial septal defect) is a rare congenital cardiac deformity, that occurs on between the inferior vena cava and right atrium. Diagnosis of inferior sinus venosus ASD is difficult because of its infero-posterior location of the fossa ovalis. Therefor, exact anatomical diagnosis by preoperative and intraoperative transesophageal echocardiography is necessary at preoperation and during the operation. We present a case of residual ASD, which was diagnosed secundum ASD and repaired when the patient was 10 years old. Residual ASD was diagnosed by cardiac echocardiography in preparation of otorhinolaryngology operation. Therefore, reoperation of residual ASD was done when the patient was 24 years old. The patient had secundum ASD and inferior sinus venosus ASD, but in the prior operation, inferior sinus venosus ASD wasn't found and only secundum ASD was repaired. In reoperation, inferior sinus venosus ASD was reveled and patch closure was done.

• Journal of Chest Surgery
• /
• v.40 no.9
• /
• pp.624-628
• /
• 2007

Between 2001 and 2006, 3 neonates that had multilevel left ventricular outflow tract obstruction and a ventricular septal defect underwent the Norwood-Rastelli procedure. The body weights ranged from 2.9 to 3.1 kg. The patients had a near normal sized mitral valve and left ventricle. We simultaneously performed a modified Norwood procedure with native tissues-to-tissue anastomosis without circulatory arrest, and a Rastelli type procedure using a non-valved conduit from the right ventricle to the pulmonary artery and intracardiac patch baffling from the left ventricle to the pulmonary valve via the ventricular septal defect. The postoperative courses were uneventful. During follow-up, there was one late mortality caused by a cardiac catheterization related complication at 7 months after surgery, One patient required a Rastelli conduit change. Two patients are doing well during a follow-up period of 1 and 5 years, respectively.

• Journal of Chest Surgery
• /
• v.38 no.3 s.248
• /
• pp.181-190
• /
• 2005

The results of biventricular repair for double outlet right ventricle have been improved in recent series. We studied the surgical and long term results for total correction of double outlet right ventricle by the type of ventricular septal defect. Material and Method: Between November 1979 and December 2003, 126 patients had biventricular repair for double outlet right ventricle. The mean age was 1.8 years (range 1$\~$44) and 86 patients ($68.3\%$) were male. We classified and studied this disease by the type of VSD. Result: The locations of VSD were subaortic in 79 ($62.7\%$), subpulmonary in 17 ($13.5\%$), doubly committed in 16 ($12.7\%$) and noncommitted in 14 ($11.1\%$). 28 patients had palliative operation before total correction and the mean interval to total correction was 41.0$\pm$45.1 months. The methods of total correction were intraventricular baffling in 37 ($29.4\%$), intraventricular baffling with patch enlargement of right ventricular outflow tract in 49 ($38.9\%$), intraventricular baffling with Rastelli procedure in 15 ($11.9\%$), arterial switch operation in 8 ($6.3\%$) and REV procedure in 4 ($3.2\%$), etc. Hospital mortality rate was $10.3\%$ (13 patients) and 25 reoperations were performed in 24 patients ($19.0\%$). The risk factors for hospital mortality and reoperation were cardiopulmonary bypass time (p=0.020) and previous palliative operation (p=0.013), respectively. Follow up was possible in 98 patients and mean follow up period was 118.9$\pm$70.7 months. The percent survival and survival for freedom from reoperation at 15 years were $82.5\%$ and $66.7\%$, respectively. The survival rate was significantly lower (p=0.003) in transposition of great artery type and remote type than in simple ventricular septal defect type and tetralogy of Fallot type, but there was no statistical differences in survival rate for freedom from reoperation. Conclusion: It is thought to be that acceptible surgical and long term results can be obtained with application of appropriate methods of repair for double outlet right ventricle.

• Journal of Chest Surgery
• /
• v.36 no.8
• /
• pp.566-575
• /
• 2003

We reviewed our 18-year surgical experience of biventricular repair for double-outlet right ventricle. Material and Method: One hundred twelve consecutive patients (80 males and 32 females) who underwent biventricular repair for double-outlet right ventricle between May 1986 and September 2002 were included. We assessed risk factors for early mortality and reoperation. Reoperation-free survival rate and actual survival rate were analysed. Result: Most common type of ventricular septal defect was subaortic (n=58, 52%) and non-committed type was second most common (n=32, 29%). Four different surgical methods were used: intraventricular baffle repair (n=71 , 63%): right ventricle to pulmonary ariery conduit interposition or REV with left ventricle to aorta baffle repair (n=24, 21 .4%): arierial switch operation with left ventricle to pulmonary artery baffle (n=14, 12.5%): Senning atrial switch operation with left ventricle to pulmonary artery baffle (n=3, 2.7%). Thirty four patients(30%) underwent palliative procedures before definite repair. Twenty three patients (21%) required reoperations. There were 12 (10.7%) early deaths and 4 late deaths. Age younger than 3 months at repair (p=0.003), cardiopulmonary bypass and aortic cross clamp time (p=0.015, p=0.067), type of operation (arterial switch operation) (p <0.001) and type of ventricular septal defect (subpulmonic type) (p=0.002) were revealed as risk factors for early death in univariate analysis, while age under 3 months was the only significant risk factor in multivariate analysis. Patients younger than 1 year of age (p=0.02), pulmonary artery angioplasty at definitive repair (p=0.024), type of ventricular septal defect (non-committed) (p=0.001), type of operation (right ventricle to pulmonary artery conduit interposition and REV operation) (p=0.028, p=0.017) were risk factors for reoperation in univariate analysis but there was no significant risk factor in multivariate analysis. Follow-up was available on 91 survivals with a mean duration of 110.8$\pm$56.4 (2~201) months. 5, 10 and 15 year survival rates were 86.5%, 85% and 85% and reoperation free survival were 85%, 71.5%, 70%. Conclusion: Age under 3 months at repair, subpulmonic ventricular septal defect and arterial switch operation were significant risk factors for early mortality. Patients with non-committed ventricular septal defect and who underwent conduit interposition or REV operation were risk factors for reoperation. With careful attention to chose best timing and surgical approach depending on morphologic characteristics, biventricular repair for double outlet right ventricle can be achieved with good long-term outcome.